P089 Research priorities in cystic fibrosis: refreshing the James Lind Alliance top ten

Objectives: In 2018 we published the top ten list of CF research priorities, developed with James Lind Alliance (JLA). We have now undertaken a refresh 10 mandated by changes in landscape through introduction CFTR modulator drugs. Methods: an online (elicitation) survey, asked community (patients, family members, clinicians and researchers) which original priorities were still important them to suggest new priorities. The sorted evidence checked create questions. second (prioritisation) survey respondents selected their from these questions ranked 15 final refreshed was decided workshop. Results: elicitation had 1370 respondents: 493 (36%) people CF(pwCF), 515 (38%) friends, 362 (26%) health Table 1(abstract: P089)1.What options are available for those not able take current modulators (including rarer mutations, eligible unable tolerate)?2.What is best way diagnose lung infection when there no sputum e.g. children on modulators?3.How can relieve gastro-intestinal symptoms, such as stomach pain, bloating nausea?4.How do manage ageing population CF?5.Is reducing negative effects antibiotics resistance risk adverse symptoms CF?6.What long-term medications modulators) CF?7.What systems outside lungs pancreatic function, liver disease, gastro-intestinal, bone density etc.?8.What effective ways simplifying treatment burden CF?9.Can genetic therapies (such gene editing, stem cell mRNA technology) be used CF?10.Is preventing related diabetes (CFRD) CF? Open table tab care professionals (HCPs) researchers, 29 countries. They identified retained suggested 971 combined similar into “umbrella” took 56 together prioritisation 1417 respondents. 17 ranking taken workshop (Table 1). Conclusions: joint process representatives updated CF. patients undergoing ETI treatment, nNO value significantly increased after several months comparison baseline. This suggests that potential non-invasive biomarker examine sinonasal inflammatory disease supports observation clinical improvement patients.